Hearing Rehabilitation in Patients With Neurofibromatosis Type 2: The Quebec's Experience With Auditory Implants.

OBJECTIVE: To review a single-center experience with hearing rehabilitation in patients with neurofibromatosis type 2 (NF2) and to describe the auditory outcomes of cochlear implants (CIs) and auditory brainstem implants (ABI) in this population. STUDY DESIGN: Retrospective case series. SETTING: Tertiary referral center. PATIENTS: Twelve adult patients with NF2 who received a CI (n = 10) and/or an ABI (n = 5) between 2000 and 2021. INTERVENTIONS: Insertion of a CI and/or an ABI in NF2 patients with bilateral vestibular schwannomas (VSs). MAIN OUTCOME MEASURES: Patients' demographic data, treatment history, hearing evolution, hearing rehabilitation methods, implant details, and auditory outcomes after implantation. RESULTS: Among those who received a CI, five patients had a stable untreated VS, one patient underwent a cochlear nerve preserving surgery, and four patients received radiotherapy treatments. Six patients became regular users of their device. The median open-set sentence recognition scores were as follows: 0.5% preoperatively, 60% at 1 year postoperatively, and 80% on the most recent audiological evaluation. All patients with an ABI were implanted concomitantly with VS surgical excision, and three of them also received radiotherapy treatments. The median open-set sentence recognition scores were as follows: 4% preoperatively, 26% at 1 year postoperatively, and 0% on the most recent evaluation. Three patients became regular ABI users. CONCLUSION: Despite major technological advances, auditory outcomes with ABIs remain deceiving. Considering the overall improvement in postoperative auditory performances provided by CIs compared with ABIs, cochlear implantation standouts as a primary mean of hearing rehabilitation in NF2 patients.

Percutaneous Bone-Anchored Hearing Implant Surgery: Do Syndromic Children Have More Adverse Perioperative Outcomes?

OBJECTIVE: To evaluate and compare perioperative outcomes of percutaneous bone-anchored hearing implant (BAHI) surgery in syndromic and nonsyndromic pediatric patients. STUDY DESIGN: Retrospective cohort study. SETTING: McGill University Health Centre in Montreal, Quebec, Canada. PATIENTS: Forty-one pediatric patients (22 syndromic, 19 nonsyndromic) who underwent percutaneous BAHI surgery between March 2008 and April 2021. INTERVENTION: Percutaneous BAHI surgery. MAIN OUTCOME MEASURES: Patient demographics (age at surgery, gender, implant laterality), operative information (American Society of Anesthesia [ASA] score, anesthesia type, surgical technique, implant/abutment characteristics), and postoperative outcomes (implant stability, soft tissue integrity, surgical revisions, implant failure). RESULTS: The most frequent syndromes among implanted patients were Treacher Collins (27.3%), Goldenhar (13.6%), Trisomy 21 (13.6%), and Nager (9.1%). Syndromic patients were more frequently given higher ASA scores: 2 ( p = 0.003) and 3 ( p = 0.014). All cases of implant extrusion were in syndromic patients: two posttraumatic and two failures to osseointegrate. Nine (40.9%) syndromic patients experienced a Holgers Grade 4 skin reaction at one of their postoperative follow-up visits as compared to 0% of nonsyndromic patients ( p < 0.001). Implant stability was similar between cohorts at all postoperative time-points, except for significantly greater nonsyndromic implant stability quotient scores at 16 weeks ( p = 0.027) and 31+ weeks ( p = 0.016). CONCLUSIONS: Percutaneous BAHI surgery is a successful rehabilitation option in syndromic patients. However, it presents a relatively higher incidence of implant extrusion and severe postoperative skin reactions as compared to nonsyndromic patients. In light of these findings, syndromic patients may be great candidates for novel transcutaneous bone conduction implants.

Trans-mastoid approach for cerebrospinal fluid leak repair.

PURPOSE OF REVIEW: To describe the technique and discuss the advantages and outcomes of the trans-mastoid approach (TMA) for temporal bone cerebrospinal fluid (CSF) leak. RECENT FINDINGS: TMA for CSF leak repair is an alternative to middle cranial fossa approach (MCFA) with less morbidity and good outcomes. SUMMARY: Persistent CSF leak in the temporal bone whether idiopathic, congenital or acquired, is an indication for surgery. TMA is a valid option for surgery, competing with MCFA in selected cases. Surgical technique consists of a standard mastoidectomy, exposure of the osteodural defect, and repairing it using multiple layers in an inlay and overlay fashion. Outcomes show low recurrence and complication rates with good hearing results. However, long-term follow-up should be made, as recurrences can be delayed.